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Clinicians' perspectives on reproductive health care services for adolescents with negative pregnancy tests
Abstracts: Poster Presentations
116
INCIDENTAL FINDMG OF A PURE DYSGERMINOMA DURJNG LAPAROSCOPIC REMOVAL OF DYSGENETIC GONADS IN A PATIENT WITH SWYER‘S SYNDROME. Kathleen F. Hemev. MD, Andrea L. Zuckemxm. MD, Department of Obstetrics and Gynecology, Tufts University New England Medical Center. Boston, MA BACKGROUND: Swyer’s syndrome is a form of pure gonadal dysgenesis characterized by e female phenotype. 46XY keiyotype, hypoplastic gonads. and P normal mullerian system. Patients with Swyer’s syndrome exhibit normal stature. but have elevated gonndotmpins, and primary pubertel delay. These patients are et increased risk for germ cell tum~ts and gonadoblartomes and should have their gonads removed prophylactically. CASE: J.B. was a IS year-old female who presented to her pediatrician with primary amenonhea. In the court of evaluation, she was found to have elevated gonadottopins. A karyotype revealed 46XY in all cell lines. A pelvic ultrasound revealed the presence of a smell uterus but no gcamds. She was referred to adolescent gynecology with the presumed diagnosis of Swycr’s syndrome for contimtation of the diagnosis and funher management. On physical exemination she WY S foot 9 inches and weighed 200 Ibs (> 95* percentiles for both height and weight). Her skin exam revealed red striae on her lower abdomen. She had Tenner Stege I breast development. Her abdomen was obese and without masses. She had Tanner 4 pubic hair. I patent hymen. and normal vagina and cervix. Bimanual examination revealed I smell “tents and no palpable adnexal masses. Laboratory studies revealed FSH 54.4 mlU/ml. LH 15.4 mlU/ml. estradiol QOpg,ml. total testestemne 30 ngidl. cwttsel 6.9 ue/dL. and DHEAS 2.9 ue’dL. No ronadal tissue could be appreciated on MRJ. She underwent laparoscopic removal of bilateral dysgenetic gonads without any complications. Findings at surgery included a hypoplastic uterus. a 2 x 2 cm left gonad and B streak gonad on the right, with normal fallopian tubes bilaterally. Pathology revealed that the left streak gonad consisted ofovarian type stmme without primordial follicles. As well, a I .2 x 0.6 cm well-delineated germ cell tumm consistent with a pure dysgetminoma was evident, without any associated gonadoblestoma. The right gonad war, poorly differentiated with no evidence of male et female gonadal rests wd no mahgnancy. The patient did well postoperatively. The patient and her family declined any futther surgery. Close follaw-up IS planned CONCLUSIONS: We descrtbe a case of Swyer‘s syndrome associated with an occult dysgerminoma that was detected following laparoscopy. Laparoscapic removal of dysgenetic gonads is a feasible method to manage pattents with gonadal dysgenesis and a Y cell line The possibility of an occult neoplasla rusts. however, and consideration should be given to frozen sectwn examinntmn of gonadal tissue at the time of surgery. ,L
I
PRECOCIOUS PUBERTY IN A 6 YEAR OLD: A CASE REPORT. Andrea L. Zuckemnn. MD and Kathleen F. Hemev, MD, Department of Obstetrics and Gynecology. Tufts University, New England Medical Center, Boston. MA BACKGROUND: Ovarian etiologies of pseudoprecocious puberty mclude benign follicular cysts as well as mal~gnem juvenile grsnulosa cell tumws (JCCT). Estmdiol levels may be elevated in both, although usually higher levels ofestradiol sre found in JGCT. In addition. mullerian inhibiting substance (MIS) and inhibin levels may be helpful in diffcrcntiatmg the diagnosis. Ul@asaund is a useful modality to evaluate rhe ovaries: however. surgical resection is often necessaty to confirm the diagnosis and exclude malignancy. CASE: L.G. is a 6 year old 7 month girl with a 5 day history of breast tenderness and enlargement and vaginal discharge She had no prepubertel bleeding. She had no htstoty of medication use/abuse. Her PMH was signicant only for anention deficit disorder. On examination she was found to have Tanner Stage 2 breasts and Tanner Stage I puharche. though her hymen appeared estrogenized. Her abdomen was nonreader with no masses appreciated. Her LHiFSH were unmeasurable, thyroid and prolsctin were normal. Her initial rsrradiol(E2) level was 689pg!ml. Her bone age was nmmal Ultrasound revenled an enlarged left ovary wtb I simple cyst measuring 5.9 x 3.2 x 49cm. MIS wss normal. Repeat ultrasound 2 weeks later revealed the right ovary wtth several small cysts and IeA ovarian cyst 3.3 x 6.4 x 4.9cm. Her breasts had grown to Tanner Stage 2-3 with persistent pain Her E2 level on the day oftbe repeat ultrasound decreased te 21 Spg/ml. An exploratory laparotomy was performed which revealed an enlarged let? ovary 6 x Scm with several 2. 3mm cysts and I large cyst. Tbe right ovary had multiple 2-imm cysts A left everian cystectomy was perfoned with tntraoperstive rupture of clear fallicular fluid. Frozen section revealed bentgn ovarian cyst. Pathology was consistent with a foliicular c>st. One week post operative her E2 level was
CLINICIANS’ PERSPECTIVES ON REPRODUCTIVE HEALTH CARE SERVICES FOR ADOLESCENTS WITH NEGATIVE PREGNANCY TESTS. ‘. ALwnMonam Lois s&ikr. PHD. RN. cs. PNP: John M. Lwenthd. MD; Polly Cromwell. MSN. RN. CPNP. Heelher Re@ds. MSN, RN, CNM Yek School ofNursing. Yale School of Medxxee. end Yale-New Hsvee Hospital, New Haven. CT. s : Althcugh ado-a rttb ttcptive pregnancy tests have been identilied as a $ch-risk sdf-identdied sewdl~ . active group at risk of ~mplenaed pregnsecies lad sexeelly _. mnsndned diseases (ST&) no iuforma~ is aveilsbk sboet climc~&’ views of the setvices pmvidcd to these tgns. The putpose of this study was to sssess the service provided te UicJlesfsms who Presented et c0mmetuty be&b care fsfilities snd hsd II wgntwe pregnmcy test ES”,t. The Of the St,@ u1 t0 id&fj’ effffihve C,iI”Gd mltepies t0 Pn”eet STIh .ltd enpleneed pregeeecin. m: Pre@eey test lo@ were kept fa I three-maetb period in the psrticipetm8 boapits cmememty. pedietrk, family plaaeing, ti s&ml-based clinics (SBCs) to &temuoc the rate of negetive ptegnaacy tests. Clirwens caring for sdokscxnts in tbe cmmwity wmpkted s survey (n=l7) to determine the .evsilsbk to teens et the time of a negative test. Three focus gmDps were conducted to detwme how cbmcmns (n-25) ~c&,wd doLsccol~ who presented for care end tti. negsttve prepnettcy tit result. w oftbe 550 pregnmcy tests perhmd. 77% were negrtwe (range= 44-Iowa). AItbou8b d s8eoctes were capable of prm’idittg comptehetwe xnices. no cleu protocol wes identifted fat sew&s pmwded to patients wvrtb 1 eegstne test Slrmulal cnntacep,,oll ues not evsdeble m the SBCs. The focus gmuPs revealed tbst teens access care m a varies ofways. Ibe depd,-of setxxes provided II the tune of s eegetzve M HLTL:depsndeet oe tbe sgency and clinician pettbmmg the test, and follow-up prwxdtues were oIlen uestmctered. Few sewtces were avulsbk to parents end psxteers. Bsmers fat t-to spprqmsto care mcluded: dd&xlty negotut,e~commueicrtieg a-l IgeeCks. lack of stsiTfrs&d to aotk wltb ndolescents. sod I Adolescents with time/fneecisI ~~lrhetttts of climctaes and sgencin. CONCLUSIONS neg*tive pre~aacy te4Ls rePresent s popeJetiocr to telgec for prevelwe cere S‘~lces need to be implemented st the ylllc level snd hteesity es dwse for teens wtb positive tests md should be comprehnwrve. wntiiual. sccesstble. teee frieedl? and accommdste the various ways teem seek reproductive care. Addttmul selnces dsn oeed to be developed to addtess the needs of tbc parcue and psmlets of teens usmg reprcductwe serwos
8Od
IMPERPORATE HYMEN WlTH COMPLETE SEPTATE UTERUS, DUPLICATED CERVIX, AND LONGlTUDINAL VAGINAL SEPTUM DIAGNOSED BY MAGNETIC RESONANCE IMAGING. M. Pfeifer. Karen Berkowitz. MD, University of Pennsylvania Medical Cente;, Philadelphia, PA. BACKGROUM): Imperforate hymen is vertical fusion defect that usually presents in adolescents with severe abdominal pain. Septate uterus with cervical duplication and longitudinal vaginal septum is a rare malformation considered to he a latenl fusion defect. The simultaneous occurrence of these distinct anomalies has not been previously reported. CASE: An 11 year old female was referred IO our institution for evaluation of two enisodes of severe abdominal oain of 2 days duration associated with n&sea and vomiting. Ultras&md and magnetic resonance imaging (MRI) evaluation prior to presentation had revealed a large ahdomino-pelvic cystic mass meawing 13 hy 8 cm felt to be consistent with hematrometra and hilateral hematosalpinges. Her physical exam was notable for Tanner 3 lx-east and pubic development and a tender pelvic mass extendinr: to the umbilicus. Pelvic exam revealed a no&patent vagina with bilge on valsalva and a cystic mass u&able on rectal exam. An MRI was obtained which revealed a larre heiatocolpos with a midline longitudinal vaginal septum extending 61 cm above the intact hymen with a complete uterine .septum and duplicated cervix. There was no evidence of hydrowlpinx. The patient was taken to he operating room where excision of the imperforate hymen was performed with drainage of 12OMl cc of accumulated old menstrual hlood. The vaginal septum was not resected. Examination revealed a complete longitudinal &&tl septum extending to jusr ahove the hymen with 2 separate cervices viaihle. Post operatively she has had no complications.
(ZOI\ICLUSIONS: The above case describes the first reported simultaneous occurrence of imperforau hymen and complete &xine septum, duplicated cervix, and longitudinal vaginal septum. MRl was instrumenti in making the correct diapnosis non-invasively in trrdcr to plan
lhr appropriate
surgical
intwvcnlwn.
116
INCIDENTAL FINDMG OF A PURE DYSGERMINOMA DURJNG LAPAROSCOPIC REMOVAL OF DYSGENETIC GONADS IN A PATIENT WITH SWYER‘S SYNDROME. Kathleen F. Hemev. MD, Andrea L. Zuckemxm. MD, Department of Obstetrics and Gynecology, Tufts University New England Medical Center. Boston, MA BACKGROUND: Swyer’s syndrome is a form of pure gonadal dysgenesis characterized by e female phenotype. 46XY keiyotype, hypoplastic gonads. and P normal mullerian system. Patients with Swyer’s syndrome exhibit normal stature. but have elevated gonndotmpins, and primary pubertel delay. These patients are et increased risk for germ cell tum~ts and gonadoblartomes and should have their gonads removed prophylactically. CASE: J.B. was a IS year-old female who presented to her pediatrician with primary amenonhea. In the court of evaluation, she was found to have elevated gonadottopins. A karyotype revealed 46XY in all cell lines. A pelvic ultrasound revealed the presence of a smell uterus but no gcamds. She was referred to adolescent gynecology with the presumed diagnosis of Swycr’s syndrome for contimtation of the diagnosis and funher management. On physical exemination she WY S foot 9 inches and weighed 200 Ibs (> 95* percentiles for both height and weight). Her skin exam revealed red striae on her lower abdomen. She had Tenner Stege I breast development. Her abdomen was obese and without masses. She had Tanner 4 pubic hair. I patent hymen. and normal vagina and cervix. Bimanual examination revealed I smell “tents and no palpable adnexal masses. Laboratory studies revealed FSH 54.4 mlU/ml. LH 15.4 mlU/ml. estradiol QOpg,ml. total testestemne 30 ngidl. cwttsel 6.9 ue/dL. and DHEAS 2.9 ue’dL. No ronadal tissue could be appreciated on MRJ. She underwent laparoscopic removal of bilateral dysgenetic gonads without any complications. Findings at surgery included a hypoplastic uterus. a 2 x 2 cm left gonad and B streak gonad on the right, with normal fallopian tubes bilaterally. Pathology revealed that the left streak gonad consisted ofovarian type stmme without primordial follicles. As well, a I .2 x 0.6 cm well-delineated germ cell tumm consistent with a pure dysgetminoma was evident, without any associated gonadoblestoma. The right gonad war, poorly differentiated with no evidence of male et female gonadal rests wd no mahgnancy. The patient did well postoperatively. The patient and her family declined any futther surgery. Close follaw-up IS planned CONCLUSIONS: We descrtbe a case of Swyer‘s syndrome associated with an occult dysgerminoma that was detected following laparoscopy. Laparoscapic removal of dysgenetic gonads is a feasible method to manage pattents with gonadal dysgenesis and a Y cell line The possibility of an occult neoplasla rusts. however, and consideration should be given to frozen sectwn examinntmn of gonadal tissue at the time of surgery. ,L
I
PRECOCIOUS PUBERTY IN A 6 YEAR OLD: A CASE REPORT. Andrea L. Zuckemnn. MD and Kathleen F. Hemev, MD, Department of Obstetrics and Gynecology. Tufts University, New England Medical Center, Boston. MA BACKGROUND: Ovarian etiologies of pseudoprecocious puberty mclude benign follicular cysts as well as mal~gnem juvenile grsnulosa cell tumws (JCCT). Estmdiol levels may be elevated in both, although usually higher levels ofestradiol sre found in JGCT. In addition. mullerian inhibiting substance (MIS) and inhibin levels may be helpful in diffcrcntiatmg the diagnosis. Ul@asaund is a useful modality to evaluate rhe ovaries: however. surgical resection is often necessaty to confirm the diagnosis and exclude malignancy. CASE: L.G. is a 6 year old 7 month girl with a 5 day history of breast tenderness and enlargement and vaginal discharge She had no prepubertel bleeding. She had no htstoty of medication use/abuse. Her PMH was signicant only for anention deficit disorder. On examination she was found to have Tanner Stage 2 breasts and Tanner Stage I puharche. though her hymen appeared estrogenized. Her abdomen was nonreader with no masses appreciated. Her LHiFSH were unmeasurable, thyroid and prolsctin were normal. Her initial rsrradiol(E2) level was 689pg!ml. Her bone age was nmmal Ultrasound revenled an enlarged left ovary wtb I simple cyst measuring 5.9 x 3.2 x 49cm. MIS wss normal. Repeat ultrasound 2 weeks later revealed the right ovary wtth several small cysts and IeA ovarian cyst 3.3 x 6.4 x 4.9cm. Her breasts had grown to Tanner Stage 2-3 with persistent pain Her E2 level on the day oftbe repeat ultrasound decreased te 21 Spg/ml. An exploratory laparotomy was performed which revealed an enlarged let? ovary 6 x Scm with several 2. 3mm cysts and I large cyst. Tbe right ovary had multiple 2-imm cysts A left everian cystectomy was perfoned with tntraoperstive rupture of clear fallicular fluid. Frozen section revealed bentgn ovarian cyst. Pathology was consistent with a foliicular c>st. One week post operative her E2 level was
CLINICIANS’ PERSPECTIVES ON REPRODUCTIVE HEALTH CARE SERVICES FOR ADOLESCENTS WITH NEGATIVE PREGNANCY TESTS. ‘. ALwnMonam Lois s&ikr. PHD. RN. cs. PNP: John M. Lwenthd. MD; Polly Cromwell. MSN. RN. CPNP. Heelher Re@ds. MSN, RN, CNM Yek School ofNursing. Yale School of Medxxee. end Yale-New Hsvee Hospital, New Haven. CT. s : Althcugh ado-a rttb ttcptive pregnancy tests have been identilied as a $ch-risk sdf-identdied sewdl~ . active group at risk of ~mplenaed pregnsecies lad sexeelly _. mnsndned diseases (ST&) no iuforma~ is aveilsbk sboet climc~&’ views of the setvices pmvidcd to these tgns. The putpose of this study was to sssess the service provided te UicJlesfsms who Presented et c0mmetuty be&b care fsfilities snd hsd II wgntwe pregnmcy test ES”,t. The Of the St,@ u1 t0 id&fj’ effffihve C,iI”Gd mltepies t0 Pn”eet STIh .ltd enpleneed pregeeecin. m: Pre@eey test lo@ were kept fa I three-maetb period in the psrticipetm8 boapits cmememty. pedietrk, family plaaeing, ti s&ml-based clinics (SBCs) to &temuoc the rate of negetive ptegnaacy tests. Clirwens caring for sdokscxnts in tbe cmmwity wmpkted s survey (n=l7) to determine the .evsilsbk to teens et the time of a negative test. Three focus gmDps were conducted to detwme how cbmcmns (n-25) ~c&,wd doLsccol~ who presented for care end tti. negsttve prepnettcy tit result. w oftbe 550 pregnmcy tests perhmd. 77% were negrtwe (range= 44-Iowa). AItbou8b d s8eoctes were capable of prm’idittg comptehetwe xnices. no cleu protocol wes identifted fat sew&s pmwded to patients wvrtb 1 eegstne test Slrmulal cnntacep,,oll ues not evsdeble m the SBCs. The focus gmuPs revealed tbst teens access care m a varies ofways. Ibe depd,-of setxxes provided II the tune of s eegetzve M HLTL:depsndeet oe tbe sgency and clinician pettbmmg the test, and follow-up prwxdtues were oIlen uestmctered. Few sewtces were avulsbk to parents end psxteers. Bsmers fat t-to spprqmsto care mcluded: dd&xlty negotut,e~commueicrtieg a-l IgeeCks. lack of stsiTfrs&d to aotk wltb ndolescents. sod I Adolescents with time/fneecisI ~~lrhetttts of climctaes and sgencin. CONCLUSIONS neg*tive pre~aacy te4Ls rePresent s popeJetiocr to telgec for prevelwe cere S‘~lces need to be implemented st the ylllc level snd hteesity es dwse for teens wtb positive tests md should be comprehnwrve. wntiiual. sccesstble. teee frieedl? and accommdste the various ways teem seek reproductive care. Addttmul selnces dsn oeed to be developed to addtess the needs of tbc parcue and psmlets of teens usmg reprcductwe serwos
8Od
IMPERPORATE HYMEN WlTH COMPLETE SEPTATE UTERUS, DUPLICATED CERVIX, AND LONGlTUDINAL VAGINAL SEPTUM DIAGNOSED BY MAGNETIC RESONANCE IMAGING. M. Pfeifer. Karen Berkowitz. MD, University of Pennsylvania Medical Cente;, Philadelphia, PA. BACKGROUM): Imperforate hymen is vertical fusion defect that usually presents in adolescents with severe abdominal pain. Septate uterus with cervical duplication and longitudinal vaginal septum is a rare malformation considered to he a latenl fusion defect. The simultaneous occurrence of these distinct anomalies has not been previously reported. CASE: An 11 year old female was referred IO our institution for evaluation of two enisodes of severe abdominal oain of 2 days duration associated with n&sea and vomiting. Ultras&md and magnetic resonance imaging (MRI) evaluation prior to presentation had revealed a large ahdomino-pelvic cystic mass meawing 13 hy 8 cm felt to be consistent with hematrometra and hilateral hematosalpinges. Her physical exam was notable for Tanner 3 lx-east and pubic development and a tender pelvic mass extendinr: to the umbilicus. Pelvic exam revealed a no&patent vagina with bilge on valsalva and a cystic mass u&able on rectal exam. An MRI was obtained which revealed a larre heiatocolpos with a midline longitudinal vaginal septum extending 61 cm above the intact hymen with a complete uterine .septum and duplicated cervix. There was no evidence of hydrowlpinx. The patient was taken to he operating room where excision of the imperforate hymen was performed with drainage of 12OMl cc of accumulated old menstrual hlood. The vaginal septum was not resected. Examination revealed a complete longitudinal &&tl septum extending to jusr ahove the hymen with 2 separate cervices viaihle. Post operatively she has had no complications.
(ZOI\ICLUSIONS: The above case describes the first reported simultaneous occurrence of imperforau hymen and complete &xine septum, duplicated cervix, and longitudinal vaginal septum. MRl was instrumenti in making the correct diapnosis non-invasively in trrdcr to plan
lhr appropriate
surgical
intwvcnlwn.